Michele D'Alto1, Roberto Badagliacca2, Francesco Lo Giudice3, Paola Argiento4, Gavino Casu5, Marco Corda6, Michele Correale7, Stefano Ghio8, Alessandra Greco8, Mariangela Lattanzio9, Valentina Mercurio10, Giuseppe Paciocco11, Silvia Papa2, Renato Prediletto12, Emanuele Romeo4, Maria Giovanna Russo13, Alessandro Tayar14, Patrizio Vitulo15, Carmine Dario Vizza2, Paolo Golino13, Robert Naeije16. 1. Department of Cardiology, Monaldi Hospital, "L. Vanvitelli" University, Naples, Italy; Italian Pulmonary Hypertension Network (iPHnet), Italy. Electronic address: mic.dalto@tin.it. 2. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiology, La Sapienza University, Rome, Italy. 3. Italian Pulmonary Hypertension Network (iPHnet), Italy; National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom. 4. Department of Cardiology, Monaldi Hospital, "L. Vanvitelli" University, Naples, Italy; Italian Pulmonary Hypertension Network (iPHnet), Italy. 5. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiology, Ospedale San Francesco, Nuoro, Italy. 6. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiology, Ospedale Brotzu, Cagliari, Italy. 7. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiology, Ospedali Riuniti University Hospital, Foggia, Italy. 8. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. 9. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Cardiovascular Disease, Fondazione Macchi ASST Sette Laghi, University of Insubria, Varese, Italy. 10. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Translational Medical Sciences, Federico II University, Naples, Italy. 11. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Pulmonology, University of Milano-Bicocca, Monza, Italy. 12. Italian Pulmonary Hypertension Network (iPHnet), Italy; Department of Pulmonology, CNR Tuscany Region Gabriele Monasterio Foundation and Institute of Clinical Physiology, Pisa, Italy. 13. Department of Cardiology, Monaldi Hospital, "L. Vanvitelli" University, Naples, Italy. 14. National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom. 15. Italian Pulmonary Hypertension Network (iPHnet), Italy; Cardiothoracic and transplant Department, IRCCS Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione (ISMETT), Palermo, Italy. 16. Department of Pathophysiology, Free University of Brussels, Brussels, Belgium.
Abstract
BACKGROUND: Upfront combination therapy with ambrisentan and tadalafil has been reported to improve the condition of patients with pulmonary arterial hypertension (PAH) more than with either drug alone. However, little is known about the long-term associated changes in hemodynamics and risk assessment scores. METHODS: This was a multicenter, retrospective analysis of clinical data in 106 patients with newly diagnosed PAH. Clinical evaluations, including demographics, medical history, World Health Organization (WHO) functional class (FC) and 6-minute walk distance (6MWD), right heart catheterization, and Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score 2.0, were assessed over 48 months of ambrisentan‒tadalafil therapy. RESULTS: At baseline, 9 patients (9%) showed a low (<7), 48 patients (45%) showed an intermediate (7-8), and 49 patients (46%) showed a high (>8) REVEAL risk score. At a median follow-up of 2 years, 45 patients (43%) showed a low, 47 patients (44%) showed an intermediate, and 14 patients (13%) showed a high REVEAL score, along with improvements in WHO FC, 6MWD and a decrease in mean pulmonary artery pressure and N-terminal pro brain natriuretic peptide (all p < 0.001). Pulmonary vascular resistance (PVR) decreased by 37% from 11.5 ± 6.5 to 7.2 ± 4.1 Wood units (p < 0.001). A total of 61 patients (57%) remained in intermediate-risk or high-risk categories. Low-risk patients had either a decrease in PVR of >50% or a stroke volume within the limits of normal. CONCLUSIONS: Initial combination therapy with ambrisentan and tadalafil in PAH improves the REVEAL risk score in proportion to decreased PVR and preserved stroke volume but still insufficiently so in approximately 50% of the patients.
BACKGROUND: Upfront combination therapy with ambrisentan and tadalafil has been reported to improve the condition of patients with pulmonary arterial hypertension (PAH) more than with either drug alone. However, little is known about the long-term associated changes in hemodynamics and risk assessment scores. METHODS: This was a multicenter, retrospective analysis of clinical data in 106 patients with newly diagnosed PAH. Clinical evaluations, including demographics, medical history, World Health Organization (WHO) functional class (FC) and 6-minute walk distance (6MWD), right heart catheterization, and Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score 2.0, were assessed over 48 months of ambrisentan‒tadalafil therapy. RESULTS: At baseline, 9 patients (9%) showed a low (<7), 48 patients (45%) showed an intermediate (7-8), and 49 patients (46%) showed a high (>8) REVEAL risk score. At a median follow-up of 2 years, 45 patients (43%) showed a low, 47 patients (44%) showed an intermediate, and 14 patients (13%) showed a high REVEAL score, along with improvements in WHO FC, 6MWD and a decrease in mean pulmonary artery pressure and N-terminal pro brain natriuretic peptide (all p < 0.001). Pulmonary vascular resistance (PVR) decreased by 37% from 11.5 ± 6.5 to 7.2 ± 4.1 Wood units (p < 0.001). A total of 61 patients (57%) remained in intermediate-risk or high-risk categories. Low-risk patients had either a decrease in PVR of >50% or a stroke volume within the limits of normal. CONCLUSIONS: Initial combination therapy with ambrisentan and tadalafil in PAH improves the REVEAL risk score in proportion to decreased PVR and preserved stroke volume but still insufficiently so in approximately 50% of the patients.
Authors: David Langleben; Stylianos E Orfanos; Benjamin D Fox; Nathan Messas; Michele Giovinazzo; John D Catravas Journal: J Clin Med Date: 2022-08-05 Impact factor: 4.964
Authors: Valentina Mercurio; Hussein J Hassan; Mario Naranjo; Alessandra Cuomo; Jeremy A Mazurek; Paul R Forfia; Aparna Balasubramanian; Catherine E Simpson; Rachel L Damico; Todd M Kolb; Stephen C Mathai; Steven Hsu; Monica Mukherjee; Paul M Hassoun Journal: J Clin Med Date: 2022-07-12 Impact factor: 4.964